A case of immunoglobulin G4-related lung disease with bilateral diffuse infiltration

Abstract

Rationale:Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a rare and chronic progressive autoimmune disease. It is a novel clinical entity characterized by elevated serum IgG4 concentration and tissue infiltration by IgG4+ plasma cells. IgG4-related lung disease (IgG4-RLD) has been described as interstitial pneumonia and inflammatory pseudotumor, with various abnormal radiographic patterns. We report a case of IgG4-related lung disease with bilateral diffuse infiltration.Patient concerns:A 65-year-old woman was admitted to our hospital because of cough, sputum, and fever. Chest computed tomography (CT) revealed multifocal areas of consolidations, nodules, and ground glass opacities in both lungs. She still had fever after anti-infective therapy.Diagnoses:Bronchial bronchoscopy and percutaneous lung biopsy were performed, resulted in a pathological diagnosis of IgG4-RLD.Interventions:The patient was started on oral prednisolone 30 mg/day for 28 days, and then was gradually tapered.Outcomes:After one week treatment, the temperature was stable, CT showed significant improvement in the areas of consolidations and nodules.Lessons:It is a typical case of IgG4-RLD. This case indicated that suggestive evidence, radiological appearances, serum tests, pathological characteristics, and classic therapy IgG4-RLD. It is a rare disease that needs our more attention in future.

IgG4‐related disease associated with the primary manifestation of recurrent cerebral venous thrombosis: A rare case report

“…IgG4‐RD is a newly recognized condition characterized by elevated serum IgG4 and tissue infiltrates of IgG4+ plasma cells. 10 IgG4‐RD has been mentioned as a systemic disease since 2001 when increased serum IgG4 concentrations were reported in patients with autoimmune pancreatitis (AIP) 11 ; later on in 2004, by learning that AIP was seen with the involvement of other organs, the term “IgG4‐related autoimmune disease” was proposed, 12 but the term “IgG4‐RD” was established later in 2012 to foregather some of the known conditions in patients with common characteristics. 13 IgG4 is proposed to unify and be the link to individual fibro‐inflammatory reactions in different organs of a patient and is found in high amounts in the serum of IgG4‐RD patients 10 , 13 (except for about 30%–50% of patients who have normal serum levels 12 ).…”

Plasma cell‐rich bronchoalveolar lavage fluid: Could be a first clue to IgG4 plasma cell‐related lung disease

“…IgG4‐related diseases encompass a spectrum of disorders with raised serum IgG4 levels and substantial infiltration by IgG4 positive plasma cells that involve different body organs either as isolated or multisystemic lesions 10 . Lung manifestations include inflammatory pseudotumor, interstitial pneumonitis, organizing pneumonia, bronchiectasis, bronchiolitis and lymphomatoid granulomatosis 5‐8,10 . Patients with organ involvement or damage consistent with IgG4‐related respiratory disease, IgG4 levels of >135 mg/dL in serum, histopathology showing marked lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, eosinophilic infiltrates, and IgG4/IgG ratio of >40% are diagnosed with IgG4‐related respiratory disease.…”

“…Patients with organ involvement or damage consistent with IgG4‐related respiratory disease, IgG4 levels of >135 mg/dL in serum, histopathology showing marked lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, eosinophilic infiltrates, and IgG4/IgG ratio of >40% are diagnosed with IgG4‐related respiratory disease. Although, an increase of IgG4 positive plasma cells is helpful for suspecting the diagnosis, elevated serum IgG4 and plasma cells may also be seen in bronchiectasis, asthma, idiopathic pulmonary fibrosis, emphysema, hypersensitivity pneumonitis, primary sclerosing cholangitis, inflammatory bowel disease, Hashimoto's thyroiditis, and in 5% of the normal population 5‐11 . The accuracy of serum IgG4 levels of >135‐144 mg/dL showed a sensitivity of 87% and specificity of 83% for diagnosing IgG4‐related respiratory disease 11 .…”

“…They include extrinsic allergic alveolitis, organizing pneumonia, and chronic eosinophilic pneumonia. Recently, reports of IgG4 plasma cell‐related diseases involving the lungs have been described mostly in tissue specimens 5‐8 . There are few reports of this diagnosis made in cytologic specimens.…”

IgG4 Related Disease associated with the primary manifestation of recurrent Cerebral Venous Thrombosis: a rare case report

“…IgG4-RD has been mentioned as a systemic disease since 2001 when increased serum IgG4 concentrations were reported in patients of autoimmune pancreatitis 16 , but the term "IgG4-RD" was established later in 2012 to foregather some of the known conditions in patients with common characteristics 17 . IgG4 is proposed to unify and be the link to individual fibro-inflammatory reactions in different organs of a patient and is found in high amounts in the serum of IgG4-RD patients 15,17 . Involvement of every organ is possible, but the nervous system is not the most commonly affected site 18 , and isolated CNS involvement is rarely reported 19 .…”